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A 72-year-old blind woman with pseudoxanthoma elasticum had typical angina pectoris for 6 weeks. She was admitted to our emergency room with signs of anterior myocardial ischemia on ECG. On physical examination there was a remarkable wrinkling of the skin at her neck and elbows.

Coronary angiography showed a significant stenosis of the left anterior descending artery (LAD) (Figure 1). Because of the eccentric configuration, an atherectomy was carried out, with good primary results (Figure 2).

Histological examination of the coronary artery specimen revealed an extensive elastolysis and fragmentation of the lamina elastica interna with microcalcifications between intima and media (Figures 3 and 4⇓). These morphological features are characteristic of a pseudoxanthoma elasticum. After atherectomy, there were no recurrent episodes of chest pain, and coronary angiography at 6-month follow-up showed a widely patent LAD.

Figure 4. Extensive elastolysis and fragmentation of the lamina elastica interna with microcalcifications between intima and media (Kossa stain; original magnification, ×400).

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.